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Rabbit Anti-Gemin 5/Biotin Conjugated antibody (bs-20266R-Bio)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-20266R-Bio
英文名称 Anti-Gemin 5/Biotin
中文名称 生物素标记的脊髓性肌萎缩症蛋白Gemin5抗体
别    名 Gemin5; Gemin-5; gem (nuclear organelle) associated protein 5; Gem-associated protein 5; GEMIN5; GEMI5_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应
产品应用 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 169kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Gemin 5
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord (1). SMA is caused by deletion or loss-of-function mutations in the SMN (Survival of Motor Neuron) gene (2). Gemin5, the protein product of human chromosome 5q33.3 (3), associates directly with SMN and is a part of the SMN complex containing Gemin2, Gemin3, Gemin4 and Gemin6 as well as several spliceosomal snRNP proteins (2,4). The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus (2). The SMN complex is found in both the cytoplasm and the nucleus (2). The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies) (2,5,6). Gemin5 interacts with several snRNP core proteins including SmB, SmD1, SmD2, SmD3 and SmE (7). The amino terminal half of Gemin5 contains 13 WD repeat domains and a coiled-coil motif near the C-terminus (7).

Function:
Gemin 5 is part of a large macromolecular complex localized to both the cytoplasm and the nucleus that plays a role in the cytoplasmic assembly of small nuclear ribonucleoproteins (snRNPs). Other members of this complex include SMN, Gemin 2 (SIP1), Gemin 3 (DDX20), and Gemin 4.

Subunit:
Part of the core SMN complex that contains SMN1, SIP1/GEMIN2, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts directly with SMN1, SNRPB, SNRPD1, SNRPD2, SNRPD3 and SNRPE.

Subcellular Location:
Nucleus, nucleoplasm. Nucleus, gem. Cytoplasm. Note=Found both in the nucleoplasm and in nuclear bodies called gems (Gemini of Cajal bodies) that are often in proximity to Cajal (coiled) bodies. Also found in the cytoplasm.

Similarity:
Belongs to the WD repeat gemin-5 family.
Contains 13 WD repeats.

Database links:

Entrez Gene: 25929 Human

Entrez Gene: 216766 Mouse

SwissProt: Q8TEQ6 Human

SwissProt: Q3UPH2 Mouse

SwissProt: Q8BX17 Mouse

Unigene: 483921 Human

Unigene: 275349 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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